454
Original article
Effective nonoperative treatment in juvenile idiopathic scoliosis Lotte van Hessema, Janneke J.P. Schimmelb, Harm C.A. Graata and Marinus de Kleuvera,c Nonoperative management of juvenile idiopathic scoliosis (JIS) has been reported to be less effective than that of infantile idiopathic scoliosis. The goal of this study was to analyse the results of casting and/or bracing in JIS. Clinical data from seven patients with JIS, treated with casting followed by bracing (n = 3) or by bracing alone (n = 4), were retrospectively collected, and curve severity was measured before, during and after treatment. The median Cobb angle decreased from 37° to 25°. No patient needed surgery at a median follow-up of 4.6 years (3.4–9.1 years). Casting and/ or bracing is effective for the management of JIS. J Pediatr
Introduction Although idiopathic scoliosis is a relatively common spinal deformity in young adolescents, it is rare in young children. A frequently used classification system subdivides patients on the basis of their age into the following categories: infantile scoliosis (0–3 years), juvenile scoliosis (3–10 years) and adolescent scoliosis (>10 years) [1]. Others prefer to use the term ‘early-onset scoliosis’ before the age of 5 years and ‘late-onset scoliosis’ after the age of 5 years, as suggested by Dickson [2]. Scoliosis is a three-dimensional deformity of the spine, the chest wall and the trunk. In a new born, the total lung volume is 6% of the adult lung volume. At the age of 5 and 10 years, the total lung volume is 30 and 50% of the adult lung volume, respectively [3]. Therefore, especially infantile and juvenile scoliosis patients are at risk of developing a serious chest wall deformity and associated restriction of lung development [2,4]. In severe cases, this might eventually lead to permanent cardiopulmonary problems [2,5,6]. An early start of treatment in young children with idiopathic scoliosis is crucial [7]. The growth rate of bones, and therefore also the spine, is most rapid in the first 2 years of life, and this growth has successfully been harnessed as a corrective force in nonoperative treatment with casting and bracing of progressive infantile scoliosis [8–10]. In the study by Mehta [8], which included scoliosis patients who were less than 4 years old, only 12.5% of the patients required a spinal fusion by the end of follow-up after casting and bracing. Smith and colleagues The work was performed at the Sint Maartenskliniek Nijmegen, The Netherlands. 1060-152X © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
Orthop B 23:454–460 © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins. Journal of Pediatric Orthopaedics B 2014, 23:454–460 Keywords: bracing, casting, juvenile idiopathic scoliosis, nonoperative scoliosis treatment Departments of aOrthopaedic Surgery, bResearch, Sint Maartenskliniek, Nijmegen and cDepartment of Orthopaedic Surgery, VU University Medical Centre, Amsterdam, The Netherlands Correspondence to Janneke J.P. Schimmel, MSc, Department of Research, Sint Maartenskliniek, P.O. Box 9011, 6500 GM Nijmegen, The Netherlands Tel: + 31 24 352 8119; fax: + 31 24 365 9154; e-mail: [email protected]
did not report any surgery rates; however, they demonstrated an average correction of 59% with casting in 10 infantile patients, with an average follow-up of 32 months (range 6–103 months). However, in this study, brace treatment alone was less effective, resulting in progression of the curve in 52% of patients [9]. In a third study, 89% of the infantile patients also had a good response to casting, with a surgery rate of 16% at final follow-up [10]. However, in the somewhat older patients with juvenile scoliosis, casting and/or bracing has been reported to be much less successful than in the infantile scoliosis population, with surgery rates ranging from 27 to 100% after nonoperative treatment [11–18]. Surgical correction of juvenile deformities can be performed by instrumented fusion or by growing rod constructs. A spinal fusion, especially under the age of 10 years, restricts longitudinal growth of the spine and thus can result in restricted lung development, cardiopulmonary problems and/or a serious crankshaft phenomenon [19]. Because of these disadvantages, growing rod systems have been used more commonly over the last 10 years, but they also have many undesired side effects (high complication rate, multiple surgeries, spontaneous autofusion) and still require a final definitive spinal fusion [9,19–21]. The ideal treatment in young children with significant remaining growth corrects the spinal and thoracic deformity, allows longitudinal growth of the spine, and preserves motion in the spine. The high incidence and severity of complications associated with surgical treatment, along with good results of nonoperative treatment in infantile scoliosis, have resulted in a renewed interest in nonoperative treatment of juvenile scoliosis. DOI: 10.1097/BPB.0000000000000077
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Nonoperative treatment in JIS van Hessem et al. 455
Fig. 1
Casting on a traction table. (a) Photograph of a patient (7 years old) on a traction table used for elongation derotation flexion casting, showing the positioning with the spine in slight flexion (the hips are flexed) and the head and hip traction applied (4–5 kg). (b) The same patient during casting; spinal derotation is applied to correct the curvature.
The natural history shows that 70–95% of curves in juvenile scoliosis progress, of which 50% eventually need (surgical) treatment [15,16,22], which justifies earlier treatment if feasible. However, the optimal nonoperative treatment regimens are relatively unknown. Therefore, we investigated the effect of casting and/or bracing in a consecutive cohort of seven patients with idiopathic juvenile scoliosis.
Methods This is a single institute consecutive patient cohort. All patients (n = 7) with progressive juvenile idiopathic scoliosis in whom treatment with casting and/or bracing was initiated from 2003 to 2010 at our institute were included. Indication for initiation of treatment was idiopathic scoliosis diagnosed between the ages of 3 and 10 years, and a Cobb angle greater than 25° and proven progression of more than 5° in two consecutive visits. Patients with scoliosis due to congenital or neuromuscular disorders were excluded. MRI or computed tomography was performed if suspicion was raised about the possibility of a nonidiopathic origin. Three patients underwent serial casting using the elongation derotation flexion (EDF) method based on the Cotrel technique (Fig. 1) [23]. To achieve more correction, in one patient, EDF casting was applied under general anaesthesia. In the other two patients with curves deemed flexible and where we judged that the child could undergo casting without anaesthesia, the cast was applied without general anaesthesia. Bracing was used in all seven patients, after initial casting (n = 3) or as a primary treatment (n = 4). The braces used were the Boston brace (Boston Brace International, Boston, Massachusetts, USA) and a Charleston type sidebending night brace (Charleston Brace Co., Ladson, South Carolina, USA). The Boston brace is a thoraco
lumbo sacral orthosis (TLSO) and is applied to patients with a double and a single C-shaped curve pattern. It has to be worn at least 18 h a day to achieve a good result [24]. The Charleston night brace is a so-called ‘side-bending brace’, which bends the trunk towards the convexity, thereby overcorrecting the curve. It should be worn at night and was only prescribed to patients with a single C-shaped scoliotic curve [25]. As the standard modules of these braces do not fit every (small) patient, some braces were custom made, based on the ‘Boston’ or ‘Charleston’ principles. Brace treatment was discontinued when the curve remained below 25° (measured on a standing radiograph without a brace) for at least 6 months and further improvement was not expected, when the end of growth was reached or when the patient refused further bracing. Patients were routinely seen at every cast exchange and every 4–6 months during brace treatment. All available serial radiographs, with and without a cast or a brace, were reviewed by one independent investigator (L.v.H.) uninvolved in the treatment of the patients. The main outcome measure was the Cobb angle [26]. The effect of treatment was expressed as an increase or decrease in the Cobb angle at the end of treatment or at final follow-up in comparison with the Cobb angle at the beginning of treatment (ΔCobb). The percentage change in the Cobb angle was also calculated. Success was defined as no progression or a decrease in the Cobb angle and no requirement for surgery during follow-up. Within success, we distinguish ‘stabilizers’, patients with a stable curve (change in Cobb angle ≤ 5°), and ‘responders’, patients with an improvement in the curve (>5° improvement in the Cobb angle). Rib vertebra angle difference (RVAD) and the phase of the rib (the extent to which the head of the rib overlaps the apical vertebra on the convex side of the curve) were measured at the beginning of treatment [27].
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
456 Journal of Pediatric Orthopaedics B 2014, Vol 23 No 5
Table 1 Patient and curve characteristics at the start of treatment for all patients with juvenile scoliosis treated by casting and/or bracing, and type of brace used Patient
Sex
Curve type
Convex side
Cobb angle
Level (apex)
Brace
1 2 3
F M F
Thoracic, single Thoracic, single Thoracic and thoracolumbar, double
R R
F
Thoracic and thoracolumbar, double
5 6 7
F F F
Thoracic, single Thoracic, single Thoracic, single
T5–T11 (T9) T7–L1 (T11) T3–T10 (T6) T11–L3 (L1) T2–T8 (T6) T9–L2 (T12) T5–L1 (T10) T6–L2 (T8) T5–T12 (T8)
Cast followed by Charleston Boston Cast followed by Boston
4
45 37 (33) 37 (28) 35 27 44 33
R R R
Boston Charleston Cast followed by Boston Charleston
F, female; L, left; M, male; R, right.
Available data on symptoms, type and duration of treatment and complications of all patients were collected until November 2013. The study was conducted in accordance with the ethical standards in the Declaration of Helsinki and approved by the hospitals’ investigational review board.
Results The median age at the start of treatment was 6.5 years (range 3.3–7.4 years; Table 1). Five patients had a single right-sided thoracic curve (Table 1). The median Cobb angle at the start of treatment was 37° (range 27°–45°; Table 2). The median follow-up was 4.6 years (3.4–9.1 years). In three patients, other possible anomalies were suspected, which were ruled out by MRI (n = 3). The median treatment duration to date was 41 months (range 25–82 months; Table 2). To date, a 100% success rate was achieved; five patients were classified as responders and two as stabilizers (Table 2 and Fig. 2). The Cobb angle decreased with a median of 42% (range − 48 to − 29%) in the responder group. Three patients had a phase 2 rib and two had an RVAD greater than 20° at the beginning of treatment (which might suggest a poor prognosis). At the start of casting or bracing, two out of seven patients had curve magnitude greater than 40°,
Table 2
suggesting an indication for surgery [28], but both responded well to nonoperative management, with a curve reduction of 48 and 45%, respectively. Four patients stopped brace treatment before the end of follow-up (T1 measurement; Table 2). In patients 1 and 2, bracing was no longer continued as the curve had improved markedly (Cobb angle < 25°) and the curves were stable at that time. In patient 4, bracing was stopped because she had reached the end of growth (double curve, Cobb angle 27° and 36°), and patient 5 chose to discontinue brace treatment by herself. All patients are still being monitored for possible progression after discontinuation of brace treatment. The follow-up after ending brace treatment ranged from 2 to 6 years for these four patients, and two of these patients (patient 1 and 2) have shown little progression since bracing was stopped, whereas the curves of the other two patients (patient 4 and 5) remained stable. Figure 3 shows the radiographs of a responder (patient 1) and a stabilizer (patient 5) before treatment, during treatment (casting and/or bracing) and 6.6 and 5.5 years, respectively, after discontinuation of treatment. One patient reported skin irritation and pressure sores as a consequence of casting. Creating a better fitting cast solved this problem. There were no other complications reported.
Data of all patients with juvenile scoliosis treated by casting and/or bracing Age at start of treatment (years)
EDF cast duration (months)
Brace duration (months)
Total orthosis duration (months)
Cobb angle T0 (deg.)
Cobb angle T1 (deg.)
Cobb angle T2 (deg.)
1 2 3a
5.6 3.3 7.0
4 – 5
25 31 50
29 31 55
13 11
4a
7.4
–
82
82
5 6 7 Median
5.2 6.7 6.5 6.5
– 3 – 4
25 38 49 38
25 41 49 41
45 37 (33) 37 (28) 35 27 44 33 37
23 23 (26) 26 (27) 36 25 24 19 25
Patient no.
(27) 36 24
19
RVAD/rib phase T0
Change in Cobb (deg.)
Change in Cobb (%)
9/2 12/1 9 and 13/1
− 22 − 14 − 11
− 48 − 37 − 29
R R R
14.7 7.9 11.7
0 and 5/1
1
2
S
16.9
−2 − 20 − 14 − 14
−7 − 45 − 42 − 37
S R R
13.4 10.1 10.7 11.7
25/2 31/2 7/1
Age at final follow-up R/S (years)
Cobb angles, rib vertebra angle difference and rib phase are given at the beginning of the treatment (T0), at the end of the treatment (T1; if completed before the end of follow-up) and at the end of follow-up (T2). R, responder; RVAD, rib vertebra angle difference; S, stabilizer a Double curves.
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Nonoperative treatment in JIS van Hessem et al. 457
In a previous study by Mehta [8], it was suggested that after the age of 2.5 years only prevention of progression could be achieved by nonoperative treatment rather than improvement of curvature. Sanders et al. [10] and Baulesh et al. [29] reported good results (improvement of the curve) with casting in a population with infantile scoliosis, in which casting was started at a mean age of 2.2 and 2.4 years, respectively [10,29]. From the current cohort study, we believe that nonoperative treatment can be just as effective in idiopathic juvenile scoliosis as in idiopathic infantile scoliosis.
Fig. 2
(a) 50 45
Cobb angle (deg.)
40 35 30 25 20 15 10 5 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 Age (years) (b) 50 45
Cobb angle (deg.)
40 35 30 25 20 15 10 5 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 Age (years) Cobb angles measured on radiographs without a cast/brace before, during and after treatment. (a) Two patients whose curve stabilized (stabilizers). (b) Five patients whose curve improved (responders). In (a) and (b), the solid lines connect the Cobb angles measured during casting/bracing treatment. Four patients have finished brace treatment and were monitored for the rest of the follow-up period. The Cobb angles measured after discontinuation of treatment are connected by double lines.
Discussion This study has shown, unlike previous studies, that nonoperative treatment including a combination of casting and bracing or bracing alone can be effective in treating juvenile idiopathic scoliosis. We have had a 100% success rate, with five patients being classified as responders and two patients as stabilizers. The median curve decrease was 42% in the responder group. At the beginning of treatment, two out of seven patients had an indication for surgery based on their curve magnitude (>40°) [28]. Up to now we have managed to prevent surgery in all patients. An independent reviewer, uninvolved in the treatment, performed all radiographic measurements, thereby diminishing bias.
Fletcher et al. [18] also studied casting followed by bracing in idiopathic and nonidiopathic juvenile patients (mean age 3.9 years, mean Cobb angle 68.8° at start of treatment), with a comparable follow-up period (5.5 years) as in this study, but they reported a disappointing 50% surgery rate. A possible explanation for this high surgery rate in contrast to our results is that they used two different casting techniques, the Risser technique and the EDF casting technique proposed by Cotrel. In the Risser technique, translational forces are used in an underarm cast, whereas in the Cotrel casting technique (as used by us), derotational forces, besides the translational forces, are used in an over the shoulder jacket. Patients casted using the Risser technique had a much higher surgery rate [18]. A recent study by Johnston and colleagues described 27 (11 idiopathic) patients with scoliosis treated with casting, starting at a mean age of 5 years. The mean Cobb angle remained stable (65.3°→64.9°) after a mean duration of treatment of 2.4 years. However, no distinction was made between infantile and juvenile patients, nor were the results of the idiopathic patients described separately from those of the patients with syndromic diagnoses [30]. In this cohort, four patients underwent successful brace treatment without prior casting. Curve stabilization was seen in two patients, and an improvement in the Cobb angle in the other two patients. These good results are in contrast to those from several studies that suggested that nonoperative treatment should always be initiated by casting to achieve good results and that juvenile scoliosis is less likely to respond to bracing [7,9,17,19]. A possible explanation is that the curves treated by bracing without prior casting in this cohort were all below 40°. This study does have some limitations. Juvenile idiopathic scoliosis is a rare disease with diversity in clinical presentation. Therefore, only small groups can be reported. No guidelines have been available on the treatment of juvenile idiopathic scoliosis, and patients were not treated according to one specific protocol. In this cohort, patients with an initial curve magnitude greater than 40° were initiated on treatment with an EDF cast, followed by brace treatment. Those with smaller curves were only treated by bracing. Two types of braces were used: for single C-shaped curves (similar to Lenke
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458 Journal of Pediatric Orthopaedics B 2014, Vol 23 No 5
Fig. 3
Radiographs of two patients before, during and after treatment with a cast and/or a brace. (a) Standing full spine radiographs (posteroanterior) of a responder (patient 1) before treatment (age 5.5 years), during casting (age 5.6 years), during bracing (Charleston night brace, age 6 years) and at final follow-up (age 14.7 years; 6.6 years after cessation of bracing). During the entire follow-up period of 8.3 years, the curve improved from 45° to 16°. (b) Standing full spine radiographs (posteroanterior) of a stabilizer (patient 5; treated solely by bracing) before treatment (age 4.9 years), during bracing (Boston brace; age 5.3 years) and at final follow-up (age 13.3 years; 5.5 years after cessation of bracing).
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Nonoperative treatment in JIS van Hessem et al. 459
1A or 1B or five curve patterns in adolescents), sidebending braces that overcorrect the curve were used, which were worn only at night, with the patient sleeping supine in the overcorrected position. For all other curve patterns, a Boston type TLSO was used, which was worn 18 h a day. A limitation of this study is its retrospective nature, which decreases the quality. Further, despite the good results to date, it is possible that the curves of some patients will progress in the coming years, as only two patients have reached skeletal maturity (>2 years after menarche) and some were still before their pubertal accelerated growth phase at final follow-up [31]. Because we want to limit radiation in these young patients, radiographs to determine skeletal age before and during treatment were not available for all patients, making comparison between patients more difficult. On the basis of the calendar age of these patients, it was assumed that only two patients had reached the end of growth at final follow-up (patient 1 and 4). Ideally, all patients should have reached skeletal maturity to be certain that the good results are maintained. However, waiting for all patients to reach skeletal maturity would mean delaying result presentation. In the current technology driven spine surgery environment, we feel that there is a publication bias towards operative treatments such as growing rods and magnetically driven growth devices. There are limited reports on nonoperative management. With this study, we would like to make the paediatric spinal specialist community aware of the possibility of successful nonoperative treatment (casting and/or bracing) of juvenile idiopathic scoliosis before considering surgery. Even if future surgery may be necessary in some of these patients, at least this technique will have allowed all of the children in this cohort to grow significantly over many years. Further, if surgery is required over the coming years, the chance of being able to perform a definitive single surgery fusion instead of multiple complex growing spine procedures is much higher than before. Trying to predict natural history and indications for nonoperative management is always a challenge. RVAD and rib phase have been shown to predict progression in infantile scoliosis [27]. In this cohort, the RVAD and rib phase were considered normal (RVAD
Original article
Effective nonoperative treatment in juvenile idiopathic scoliosis Lotte van Hessema, Janneke J.P. Schimmelb, Harm C.A. Graata and Marinus de Kleuvera,c Nonoperative management of juvenile idiopathic scoliosis (JIS) has been reported to be less effective than that of infantile idiopathic scoliosis. The goal of this study was to analyse the results of casting and/or bracing in JIS. Clinical data from seven patients with JIS, treated with casting followed by bracing (n = 3) or by bracing alone (n = 4), were retrospectively collected, and curve severity was measured before, during and after treatment. The median Cobb angle decreased from 37° to 25°. No patient needed surgery at a median follow-up of 4.6 years (3.4–9.1 years). Casting and/ or bracing is effective for the management of JIS. J Pediatr
Introduction Although idiopathic scoliosis is a relatively common spinal deformity in young adolescents, it is rare in young children. A frequently used classification system subdivides patients on the basis of their age into the following categories: infantile scoliosis (0–3 years), juvenile scoliosis (3–10 years) and adolescent scoliosis (>10 years) [1]. Others prefer to use the term ‘early-onset scoliosis’ before the age of 5 years and ‘late-onset scoliosis’ after the age of 5 years, as suggested by Dickson [2]. Scoliosis is a three-dimensional deformity of the spine, the chest wall and the trunk. In a new born, the total lung volume is 6% of the adult lung volume. At the age of 5 and 10 years, the total lung volume is 30 and 50% of the adult lung volume, respectively [3]. Therefore, especially infantile and juvenile scoliosis patients are at risk of developing a serious chest wall deformity and associated restriction of lung development [2,4]. In severe cases, this might eventually lead to permanent cardiopulmonary problems [2,5,6]. An early start of treatment in young children with idiopathic scoliosis is crucial [7]. The growth rate of bones, and therefore also the spine, is most rapid in the first 2 years of life, and this growth has successfully been harnessed as a corrective force in nonoperative treatment with casting and bracing of progressive infantile scoliosis [8–10]. In the study by Mehta [8], which included scoliosis patients who were less than 4 years old, only 12.5% of the patients required a spinal fusion by the end of follow-up after casting and bracing. Smith and colleagues The work was performed at the Sint Maartenskliniek Nijmegen, The Netherlands. 1060-152X © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
Orthop B 23:454–460 © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins. Journal of Pediatric Orthopaedics B 2014, 23:454–460 Keywords: bracing, casting, juvenile idiopathic scoliosis, nonoperative scoliosis treatment Departments of aOrthopaedic Surgery, bResearch, Sint Maartenskliniek, Nijmegen and cDepartment of Orthopaedic Surgery, VU University Medical Centre, Amsterdam, The Netherlands Correspondence to Janneke J.P. Schimmel, MSc, Department of Research, Sint Maartenskliniek, P.O. Box 9011, 6500 GM Nijmegen, The Netherlands Tel: + 31 24 352 8119; fax: + 31 24 365 9154; e-mail: [email protected]
did not report any surgery rates; however, they demonstrated an average correction of 59% with casting in 10 infantile patients, with an average follow-up of 32 months (range 6–103 months). However, in this study, brace treatment alone was less effective, resulting in progression of the curve in 52% of patients [9]. In a third study, 89% of the infantile patients also had a good response to casting, with a surgery rate of 16% at final follow-up [10]. However, in the somewhat older patients with juvenile scoliosis, casting and/or bracing has been reported to be much less successful than in the infantile scoliosis population, with surgery rates ranging from 27 to 100% after nonoperative treatment [11–18]. Surgical correction of juvenile deformities can be performed by instrumented fusion or by growing rod constructs. A spinal fusion, especially under the age of 10 years, restricts longitudinal growth of the spine and thus can result in restricted lung development, cardiopulmonary problems and/or a serious crankshaft phenomenon [19]. Because of these disadvantages, growing rod systems have been used more commonly over the last 10 years, but they also have many undesired side effects (high complication rate, multiple surgeries, spontaneous autofusion) and still require a final definitive spinal fusion [9,19–21]. The ideal treatment in young children with significant remaining growth corrects the spinal and thoracic deformity, allows longitudinal growth of the spine, and preserves motion in the spine. The high incidence and severity of complications associated with surgical treatment, along with good results of nonoperative treatment in infantile scoliosis, have resulted in a renewed interest in nonoperative treatment of juvenile scoliosis. DOI: 10.1097/BPB.0000000000000077
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Nonoperative treatment in JIS van Hessem et al. 455
Fig. 1
Casting on a traction table. (a) Photograph of a patient (7 years old) on a traction table used for elongation derotation flexion casting, showing the positioning with the spine in slight flexion (the hips are flexed) and the head and hip traction applied (4–5 kg). (b) The same patient during casting; spinal derotation is applied to correct the curvature.
The natural history shows that 70–95% of curves in juvenile scoliosis progress, of which 50% eventually need (surgical) treatment [15,16,22], which justifies earlier treatment if feasible. However, the optimal nonoperative treatment regimens are relatively unknown. Therefore, we investigated the effect of casting and/or bracing in a consecutive cohort of seven patients with idiopathic juvenile scoliosis.
Methods This is a single institute consecutive patient cohort. All patients (n = 7) with progressive juvenile idiopathic scoliosis in whom treatment with casting and/or bracing was initiated from 2003 to 2010 at our institute were included. Indication for initiation of treatment was idiopathic scoliosis diagnosed between the ages of 3 and 10 years, and a Cobb angle greater than 25° and proven progression of more than 5° in two consecutive visits. Patients with scoliosis due to congenital or neuromuscular disorders were excluded. MRI or computed tomography was performed if suspicion was raised about the possibility of a nonidiopathic origin. Three patients underwent serial casting using the elongation derotation flexion (EDF) method based on the Cotrel technique (Fig. 1) [23]. To achieve more correction, in one patient, EDF casting was applied under general anaesthesia. In the other two patients with curves deemed flexible and where we judged that the child could undergo casting without anaesthesia, the cast was applied without general anaesthesia. Bracing was used in all seven patients, after initial casting (n = 3) or as a primary treatment (n = 4). The braces used were the Boston brace (Boston Brace International, Boston, Massachusetts, USA) and a Charleston type sidebending night brace (Charleston Brace Co., Ladson, South Carolina, USA). The Boston brace is a thoraco
lumbo sacral orthosis (TLSO) and is applied to patients with a double and a single C-shaped curve pattern. It has to be worn at least 18 h a day to achieve a good result [24]. The Charleston night brace is a so-called ‘side-bending brace’, which bends the trunk towards the convexity, thereby overcorrecting the curve. It should be worn at night and was only prescribed to patients with a single C-shaped scoliotic curve [25]. As the standard modules of these braces do not fit every (small) patient, some braces were custom made, based on the ‘Boston’ or ‘Charleston’ principles. Brace treatment was discontinued when the curve remained below 25° (measured on a standing radiograph without a brace) for at least 6 months and further improvement was not expected, when the end of growth was reached or when the patient refused further bracing. Patients were routinely seen at every cast exchange and every 4–6 months during brace treatment. All available serial radiographs, with and without a cast or a brace, were reviewed by one independent investigator (L.v.H.) uninvolved in the treatment of the patients. The main outcome measure was the Cobb angle [26]. The effect of treatment was expressed as an increase or decrease in the Cobb angle at the end of treatment or at final follow-up in comparison with the Cobb angle at the beginning of treatment (ΔCobb). The percentage change in the Cobb angle was also calculated. Success was defined as no progression or a decrease in the Cobb angle and no requirement for surgery during follow-up. Within success, we distinguish ‘stabilizers’, patients with a stable curve (change in Cobb angle ≤ 5°), and ‘responders’, patients with an improvement in the curve (>5° improvement in the Cobb angle). Rib vertebra angle difference (RVAD) and the phase of the rib (the extent to which the head of the rib overlaps the apical vertebra on the convex side of the curve) were measured at the beginning of treatment [27].
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
456 Journal of Pediatric Orthopaedics B 2014, Vol 23 No 5
Table 1 Patient and curve characteristics at the start of treatment for all patients with juvenile scoliosis treated by casting and/or bracing, and type of brace used Patient
Sex
Curve type
Convex side
Cobb angle
Level (apex)
Brace
1 2 3
F M F
Thoracic, single Thoracic, single Thoracic and thoracolumbar, double
R R
F
Thoracic and thoracolumbar, double
5 6 7
F F F
Thoracic, single Thoracic, single Thoracic, single
T5–T11 (T9) T7–L1 (T11) T3–T10 (T6) T11–L3 (L1) T2–T8 (T6) T9–L2 (T12) T5–L1 (T10) T6–L2 (T8) T5–T12 (T8)
Cast followed by Charleston Boston Cast followed by Boston
4
45 37 (33) 37 (28) 35 27 44 33
R R R
Boston Charleston Cast followed by Boston Charleston
F, female; L, left; M, male; R, right.
Available data on symptoms, type and duration of treatment and complications of all patients were collected until November 2013. The study was conducted in accordance with the ethical standards in the Declaration of Helsinki and approved by the hospitals’ investigational review board.
Results The median age at the start of treatment was 6.5 years (range 3.3–7.4 years; Table 1). Five patients had a single right-sided thoracic curve (Table 1). The median Cobb angle at the start of treatment was 37° (range 27°–45°; Table 2). The median follow-up was 4.6 years (3.4–9.1 years). In three patients, other possible anomalies were suspected, which were ruled out by MRI (n = 3). The median treatment duration to date was 41 months (range 25–82 months; Table 2). To date, a 100% success rate was achieved; five patients were classified as responders and two as stabilizers (Table 2 and Fig. 2). The Cobb angle decreased with a median of 42% (range − 48 to − 29%) in the responder group. Three patients had a phase 2 rib and two had an RVAD greater than 20° at the beginning of treatment (which might suggest a poor prognosis). At the start of casting or bracing, two out of seven patients had curve magnitude greater than 40°,
Table 2
suggesting an indication for surgery [28], but both responded well to nonoperative management, with a curve reduction of 48 and 45%, respectively. Four patients stopped brace treatment before the end of follow-up (T1 measurement; Table 2). In patients 1 and 2, bracing was no longer continued as the curve had improved markedly (Cobb angle < 25°) and the curves were stable at that time. In patient 4, bracing was stopped because she had reached the end of growth (double curve, Cobb angle 27° and 36°), and patient 5 chose to discontinue brace treatment by herself. All patients are still being monitored for possible progression after discontinuation of brace treatment. The follow-up after ending brace treatment ranged from 2 to 6 years for these four patients, and two of these patients (patient 1 and 2) have shown little progression since bracing was stopped, whereas the curves of the other two patients (patient 4 and 5) remained stable. Figure 3 shows the radiographs of a responder (patient 1) and a stabilizer (patient 5) before treatment, during treatment (casting and/or bracing) and 6.6 and 5.5 years, respectively, after discontinuation of treatment. One patient reported skin irritation and pressure sores as a consequence of casting. Creating a better fitting cast solved this problem. There were no other complications reported.
Data of all patients with juvenile scoliosis treated by casting and/or bracing Age at start of treatment (years)
EDF cast duration (months)
Brace duration (months)
Total orthosis duration (months)
Cobb angle T0 (deg.)
Cobb angle T1 (deg.)
Cobb angle T2 (deg.)
1 2 3a
5.6 3.3 7.0
4 – 5
25 31 50
29 31 55
13 11
4a
7.4
–
82
82
5 6 7 Median
5.2 6.7 6.5 6.5
– 3 – 4
25 38 49 38
25 41 49 41
45 37 (33) 37 (28) 35 27 44 33 37
23 23 (26) 26 (27) 36 25 24 19 25
Patient no.
(27) 36 24
19
RVAD/rib phase T0
Change in Cobb (deg.)
Change in Cobb (%)
9/2 12/1 9 and 13/1
− 22 − 14 − 11
− 48 − 37 − 29
R R R
14.7 7.9 11.7
0 and 5/1
1
2
S
16.9
−2 − 20 − 14 − 14
−7 − 45 − 42 − 37
S R R
13.4 10.1 10.7 11.7
25/2 31/2 7/1
Age at final follow-up R/S (years)
Cobb angles, rib vertebra angle difference and rib phase are given at the beginning of the treatment (T0), at the end of the treatment (T1; if completed before the end of follow-up) and at the end of follow-up (T2). R, responder; RVAD, rib vertebra angle difference; S, stabilizer a Double curves.
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Nonoperative treatment in JIS van Hessem et al. 457
In a previous study by Mehta [8], it was suggested that after the age of 2.5 years only prevention of progression could be achieved by nonoperative treatment rather than improvement of curvature. Sanders et al. [10] and Baulesh et al. [29] reported good results (improvement of the curve) with casting in a population with infantile scoliosis, in which casting was started at a mean age of 2.2 and 2.4 years, respectively [10,29]. From the current cohort study, we believe that nonoperative treatment can be just as effective in idiopathic juvenile scoliosis as in idiopathic infantile scoliosis.
Fig. 2
(a) 50 45
Cobb angle (deg.)
40 35 30 25 20 15 10 5 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 Age (years) (b) 50 45
Cobb angle (deg.)
40 35 30 25 20 15 10 5 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 Age (years) Cobb angles measured on radiographs without a cast/brace before, during and after treatment. (a) Two patients whose curve stabilized (stabilizers). (b) Five patients whose curve improved (responders). In (a) and (b), the solid lines connect the Cobb angles measured during casting/bracing treatment. Four patients have finished brace treatment and were monitored for the rest of the follow-up period. The Cobb angles measured after discontinuation of treatment are connected by double lines.
Discussion This study has shown, unlike previous studies, that nonoperative treatment including a combination of casting and bracing or bracing alone can be effective in treating juvenile idiopathic scoliosis. We have had a 100% success rate, with five patients being classified as responders and two patients as stabilizers. The median curve decrease was 42% in the responder group. At the beginning of treatment, two out of seven patients had an indication for surgery based on their curve magnitude (>40°) [28]. Up to now we have managed to prevent surgery in all patients. An independent reviewer, uninvolved in the treatment, performed all radiographic measurements, thereby diminishing bias.
Fletcher et al. [18] also studied casting followed by bracing in idiopathic and nonidiopathic juvenile patients (mean age 3.9 years, mean Cobb angle 68.8° at start of treatment), with a comparable follow-up period (5.5 years) as in this study, but they reported a disappointing 50% surgery rate. A possible explanation for this high surgery rate in contrast to our results is that they used two different casting techniques, the Risser technique and the EDF casting technique proposed by Cotrel. In the Risser technique, translational forces are used in an underarm cast, whereas in the Cotrel casting technique (as used by us), derotational forces, besides the translational forces, are used in an over the shoulder jacket. Patients casted using the Risser technique had a much higher surgery rate [18]. A recent study by Johnston and colleagues described 27 (11 idiopathic) patients with scoliosis treated with casting, starting at a mean age of 5 years. The mean Cobb angle remained stable (65.3°→64.9°) after a mean duration of treatment of 2.4 years. However, no distinction was made between infantile and juvenile patients, nor were the results of the idiopathic patients described separately from those of the patients with syndromic diagnoses [30]. In this cohort, four patients underwent successful brace treatment without prior casting. Curve stabilization was seen in two patients, and an improvement in the Cobb angle in the other two patients. These good results are in contrast to those from several studies that suggested that nonoperative treatment should always be initiated by casting to achieve good results and that juvenile scoliosis is less likely to respond to bracing [7,9,17,19]. A possible explanation is that the curves treated by bracing without prior casting in this cohort were all below 40°. This study does have some limitations. Juvenile idiopathic scoliosis is a rare disease with diversity in clinical presentation. Therefore, only small groups can be reported. No guidelines have been available on the treatment of juvenile idiopathic scoliosis, and patients were not treated according to one specific protocol. In this cohort, patients with an initial curve magnitude greater than 40° were initiated on treatment with an EDF cast, followed by brace treatment. Those with smaller curves were only treated by bracing. Two types of braces were used: for single C-shaped curves (similar to Lenke
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458 Journal of Pediatric Orthopaedics B 2014, Vol 23 No 5
Fig. 3
Radiographs of two patients before, during and after treatment with a cast and/or a brace. (a) Standing full spine radiographs (posteroanterior) of a responder (patient 1) before treatment (age 5.5 years), during casting (age 5.6 years), during bracing (Charleston night brace, age 6 years) and at final follow-up (age 14.7 years; 6.6 years after cessation of bracing). During the entire follow-up period of 8.3 years, the curve improved from 45° to 16°. (b) Standing full spine radiographs (posteroanterior) of a stabilizer (patient 5; treated solely by bracing) before treatment (age 4.9 years), during bracing (Boston brace; age 5.3 years) and at final follow-up (age 13.3 years; 5.5 years after cessation of bracing).
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Nonoperative treatment in JIS van Hessem et al. 459
1A or 1B or five curve patterns in adolescents), sidebending braces that overcorrect the curve were used, which were worn only at night, with the patient sleeping supine in the overcorrected position. For all other curve patterns, a Boston type TLSO was used, which was worn 18 h a day. A limitation of this study is its retrospective nature, which decreases the quality. Further, despite the good results to date, it is possible that the curves of some patients will progress in the coming years, as only two patients have reached skeletal maturity (>2 years after menarche) and some were still before their pubertal accelerated growth phase at final follow-up [31]. Because we want to limit radiation in these young patients, radiographs to determine skeletal age before and during treatment were not available for all patients, making comparison between patients more difficult. On the basis of the calendar age of these patients, it was assumed that only two patients had reached the end of growth at final follow-up (patient 1 and 4). Ideally, all patients should have reached skeletal maturity to be certain that the good results are maintained. However, waiting for all patients to reach skeletal maturity would mean delaying result presentation. In the current technology driven spine surgery environment, we feel that there is a publication bias towards operative treatments such as growing rods and magnetically driven growth devices. There are limited reports on nonoperative management. With this study, we would like to make the paediatric spinal specialist community aware of the possibility of successful nonoperative treatment (casting and/or bracing) of juvenile idiopathic scoliosis before considering surgery. Even if future surgery may be necessary in some of these patients, at least this technique will have allowed all of the children in this cohort to grow significantly over many years. Further, if surgery is required over the coming years, the chance of being able to perform a definitive single surgery fusion instead of multiple complex growing spine procedures is much higher than before. Trying to predict natural history and indications for nonoperative management is always a challenge. RVAD and rib phase have been shown to predict progression in infantile scoliosis [27]. In this cohort, the RVAD and rib phase were considered normal (RVAD
